Case 1 presentation: hemorrhagic alveolitis in systemic lupus erythematosus

Hemorrhagic alveolitis (HA) is a potentially serious complication of systemic lupus erythematosus (SLE), occurring in 1-5.4% of patients. In those presenting this condition, the prognosis is severe: most studies report mortality rates ranging from 23% to 92%, and only three case series of SLE-associated HA have described survival rates ≥50%. Here we report a case of SLE complicated with HA observed in the Rheumatology Division of the Sapienza University of Rome, Italy. The patient, a 36-year-old Caucasian woman, had been diagnosed with SLE in 2003 on the basis of photosensitivity, malar rash, polyarthritis, oral ulcers, Raynaud's phenomenon, and positive tests for antinuclear antibodies (+++, homogeneous pattern), anti-double-stranded DNA antibodies, and lupus anticoagulant; she also had low C4 and high erythrocyte sedimentation rate levels. The patient was started on prednisone 5 mg per day, hydroxychloroquine 400 mg per day, and low-dose aspirin daily, which relieved her symptoms until February 2007. At that time, she was referred to a community hospital due to recent development of dyspnea on mild exertion, cough, hemoptysis, and fever. Laboratory workup showed anemia (hemoglobin: 6.5 g/dL; hematocrit: 19.6%), while at the physical examination she presented tachycardia (140 beats per minute) and reduced vesicular murmur at the right lung base. This clinical picture, together with the presence of a shadowing at the right base posteriorly in the chest X-ray, led to the diagnosis of pneumonia, and the patient was discharged with antibiotic treatment. However, due to the persistence of anemia and the remark of weight loss, she was subsequently admitted to our hospital where received 4 transfusions of concentrated red blood cells. During the hospitalization the patient was also treated with high-dose glucocorticoids and, while excluding other potential sources of bleeding, a close examination of the lungs was made through the performance of chest high resolution computed tomography (HRCT, showing ''multiple areas of parenchymal consolidation with reticulonodular and ground glass pattern''). The chest HRCT was repeated after 1 month due to recurrent episodes of hemoptysis which showed ''a massive alveolar involvement probably related to hemorrhage''. The patient was diagnosed as HA due to SLE and undergone treatment with pulse cyclophosphamide (3 infusions of 500 mg each given 2 weeks apart) and 6 sessions of plasmapheresis. A fairly good control of the disease was achieved with a progressive raise in the hemoglobin levels, and she was discharged home after 2 months. Other sessions of cyclophosphamide and plasmapheresis were scheduled in an outpatient setting up until a full recovery. At her last visit, on March 2011, the patient was described as being still free of pulmonary symptoms while taking low-dose glucocorticoids. Earlier reports defined the presentation of AH as usually characterized by the triad of hemoptysis, abrupt fall in hemoglobin level, and new, often bilateral, pulmonary infiltrates. Indeed, this presentation was not uniformly seen in the subsequent series, and also in our patient the occurrence of fever and unilateral densities at chest X-ray made initially the diagnosis of pneumonia more likely. However, once clarified the nature of the manifestations, the treatment was found to be successful, confirming the observations by some Authors that HA is a survivable complication of SLE. References: Santos-Ocampo AS, et al. Alveolar hemorrhage in systemic lupus erythematosus: presentation and management. Chest 2000; 118: 1083-90. Badsha H, et al. Pulmonary hemorrhage in systemic lupus erythematosus. Semin Arthritis Rheum 2003; 33: 414-21.